Background Transthoracic echocardiography (TTE) is well validated for initial assessment of connective tissue disease patients with suspected pulmonary arterial hypertension (PAH). However, in patients with PAH confirmed by the gold-standard method of right heart catheterization (RHC) the role of TTE in their follow-up is less known.
Objectives To test the hypothesis that TTE can replace follow-up RHC in connective tissue disease-associated PAH.
Methods This retrospective study included 30 consecutive patients with systemic sclerosis (n=24) and mixed connective tissue disease (n=6) (mean age±SD: 60±12 years, 87% women), in whom PAH was suggested by TTE and further confirmed by a baseline RHC [pulmonary artery systolic pressure (PASP): 56.8±19.1, range 25–90mmHg; pulmonary vascular resistance (PVR): 5.9±3.8, range 0.7–14.5 Wood units; cardiac output: 4.4±1.3, range 2–7.8 L/min). All 30 patients underwent a second RHC and TTE at follow-up, after 11±6 (range 4–29) months. Ten patients had a 3rd follow-up RHC and TTE 22±7 (range 15–37) months from baseline, thus producing in all 50 pairs of baseline and follow-up measurements. By considering follow-up RHC as the gold-standard, we examined whether clinically meaningful hemodynamic changes (i.e.>15% change from baseline) in either RHC-derived PASP or PVR could be predicted by the corresponding changes from baseline in follow-up TTE. RHC and TTE were always performed, each, by the same examiner.
Results In 68% of comparisons between baseline and follow-up, the latter TTE measurements could safely replace RHC in terms of PASP estimation. Using Mc Nemars test we confirmed that the two methods did not differ significantly (Table 1). When in addition to changes in PASP, PVR changes were also considered, follow-up TTE could again safely replace the second RHC in 70% patient retests (Table 2). Of note, baseline hemodynamic values or TTE measurements did not differ between patients in whom TTE could replace RHC and those in whom the results of the two methods at follow-up were divergent.
Conclusions In a study where operator-dependent methodological errors are limited we found that the majority of connective tissue disease patients with PAH can be safely monitored in the long-term by TTE. Further studies to help identify those patients at need for follow-up RHC are warranted.
Disclosure of Interest None declared
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